NEW YORK (Reuters Health) - The progression of liver disease in patients with primary biliary cirrhosis associated with systemic sclerosis appears to be slower than the disease progression in patients with primary biliary cirrhosis alone, UK researchers report.

Dr. Andrew K. Burroughs of Royal Free Hospital, London and colleagues note that primary biliary cirrhosis often occurs in association with other autoimmune disorders. Previous research suggests primary biliary cirrhosis is associated with systemic sclerosis in 3% to 50% of cases. However, it is unclear if the prognosis of the two conditions is similar.

To investigate, the researchers used serum bilirubin concentration at the initial hospital visit to match 43 patients with primary biliary cirrhosis-systemic sclerosis to 82 with primary biliary cirrhosis alone. Of the primary biliary cirrhosis-systemic sclerosis patients, 40 had limited systemic sclerosis and 24 had systemic sclerosis diagnosed before primary biliary cirrhosis. Median follow-up was similar for both groups.

After adjustment for confounding factors, the risk of transplantation or death was significantly lower in primary biliary cirrhosis-systemic sclerosis patients (hazard ratio 0.1), according to the study findings, published in the March issue of Gut.

Overall survival was similar. There were nine deaths, with two liver-related in the combination group. In the primary biliary cirrhosis group, there were also nine deaths, of which six were liver related.

Given these findings, the researchers conclude that prognostic models for primary biliary cirrhosis alone may not be applicable to the condition associated with systemic sclerosis or other autoimmune diseases and that new models may have to be developed.